Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options

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• 作者：Gerhard J Molderings (1)
  Stefan Brettner (2)
  Jürgen Homann (3)
  Lawrence B Afrin (4)
  
• 刊名：Journal of Hematology & Oncology
• 出版年：2011
• 出版时间：December 2011
• 年：2011
• 卷：4
• 期：1
• 全文大小：496KB
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• 作者单位：Gerhard J Molderings (1)
  Stefan Brettner (2)
  Jürgen Homann (3)
  Lawrence B Afrin (4)
  
  1. Institute of Human Genetics, University Hospital of Bonn, Sigmund-Freud-Str. 25, D-53127, Bonn, Germany
  2. Department of Oncology, Hematology and Palliative Care, Kreiskrankenhaus Waldbr?l, Dr.-Goldenburgen-Str. 10, D-51545, Waldbr?l, Germany
  3. Department of Internal Medicine, Evangelische Kliniken Bonn, Waldkrankenhaus, Waldstrasse 73, D-53177, Bonn, Germany
  4. Division of Hematology/Oncology, Medical University of South Carolina, Charleston, South Carolina, USA
  
• ISSN：1756-8722

文摘

Mast cell activation disease comprises disorders characterized by accumulation of genetically altered mast cells and/or abnormal release of these cells' mediators, affecting functions in potentially every organ system, often without causing abnormalities in routine laboratory or radiologic testing. In most cases of mast cell activation disease, diagnosis is possible by relatively non-invasive investigation. Effective therapy often consists simply of antihistamines and mast cell membrane-stabilising compounds supplemented with medications targeted at specific symptoms and complications. Mast cell activation disease is now appreciated to likely be considerably prevalent and thus should be considered routinely in the differential diagnosis of patients with chronic multisystem polymorbidity or patients in whom a definitively diagnosed major illness does not well account for the entirety of the patient's presentation.